Management of G6PD Hemolysis
The management of G6PD hemolysis requires immediate discontinuation of the offending oxidant drug, supportive care, and in severe cases, blood transfusion or exchange transfusion. 1, 2
Immediate Management
Identification and Removal of Trigger
- Immediately discontinue any suspected oxidant medications or foods (particularly fava beans) 1, 2
- Common medication triggers include:
- Antimalarials (primaquine, chloroquine in high doses)
- Antibiotics (dapsone, sulfonamides, nitrofurantoin)
- Analgesics (phenazopyridine)
- Rasburicase
- Methylene blue (contraindicated in G6PD deficiency) 1
Assessment of Severity
- Laboratory evaluation:
- Complete blood count with peripheral smear (look for Heinz bodies)
- Reticulocyte count
- LDH, haptoglobin, and bilirubin (direct and indirect)
- Urinalysis (for hemoglobinuria)
- Renal function tests 1
Severity-Based Management
Mild Hemolysis (G1)
- Close clinical follow-up with serial hemoglobin monitoring
- Adequate hydration to prevent renal injury
- Monitor for worsening symptoms 1
Moderate Hemolysis (G2)
- Consider hospital admission for monitoring
- IV hydration to maintain good urine output
- Consider prednisone 0.5-1 mg/kg/day if hemolysis is significant 1
Severe Hemolysis (G3-G4)
- Hospital admission required
- IV hydration with close monitoring of urine output
- Blood transfusion if hemoglobin drops below 7-8 g/dL or if symptomatic anemia
- Prednisone 1-2 mg/kg/day (oral or IV depending on severity)
- Folic acid supplementation (1 mg daily)
- Monitor for acute kidney injury from hemoglobinuria 1
Life-Threatening Hemolysis
- Exchange transfusion should be considered in cases refractory to conventional management
- Hemodialysis may be required if acute kidney injury develops
- Intensive care monitoring for multiorgan dysfunction 1
Special Considerations
Methemoglobinemia with G6PD Deficiency
- Avoid methylene blue as it can worsen hemolysis and is ineffective in G6PD deficiency
- Use ascorbic acid (0.2-1.0 g/day orally in divided doses) as alternative treatment
- Consider exchange transfusion or hyperbaric oxygen therapy in severe cases 1
Malaria Treatment in G6PD Deficiency
- Avoid primaquine in patients with severe G6PD deficiency
- For P. vivax or P. ovale infections requiring radical cure:
- In mild-moderate G6PD deficiency (non-Mediterranean variant): consider weekly primaquine (0.75 mg base/kg, maximum 45 mg) for 8 weeks with close monitoring
- In severe G6PD deficiency: avoid 8-aminoquinolines completely 1
- For acute malaria treatment, use artemisinin-based combination therapies (ACTs) which are generally safe in G6PD deficiency 1
Prevention of Future Episodes
- G6PD testing should be performed before initiating oxidant drugs, especially in high-risk populations (African, Mediterranean, Middle Eastern, South-East Asian descent) 1, 2
- Patient education about avoiding oxidant drugs and foods
- Medical alert identification for patients with G6PD deficiency
- Genetic counseling for family members 1
Follow-up
- Monitor hemoglobin levels weekly until stable and steroid tapering is complete
- Continue folic acid supplementation during recovery phase
- Assess for complications like chronic kidney disease in severe cases
- Screen first-degree relatives for G6PD deficiency 1
Important Caveats
- The Mediterranean variant (B-) of G6PD deficiency is associated with more severe hemolytic reactions than the African variant (A-) 1, 2
- Recent evidence suggests some medications previously thought to be high-risk may be safer than expected, but caution is still warranted 3
- Infections and acute HIV can also trigger hemolysis in G6PD-deficient patients, not just medications 4
- Antioxidant medications (like dexmedetomidine) may be preferred when anesthesia is required 5